AB1279 POOR QUALITY OF LIFE AND REDUCED WORK PRODUCTIVITY IN EUROPEAN PATIENTS WITH DERMATOMYOSITIS AND POLYMYOSITIS: FINDINGS FROM A SYSTEMATIC LITERATURE REVIEW
Authors: J. Schmidt, K. Pisarczyk, R. Leff, K. Palaniswamy, E. Park, L. Long
Journal: Annals of the Rheumatic Diseases (2022)

Background Dermatomyositis (DM) and polymyositis (PM) are rare heterogenous systemic autoimmune disorders of the skin, muscles, and other organs with few effective treatment options available. They are described as devastating diseases but the full impact on patients’ lives in Europe is not well understood. Objectives To systematically review and summarize evidence on humanistic burden of disease in patients with DM and PM in Europe to better understand patient-relevant aspects of disease and key domains of life impacted by DM and PM in the European setting. Methods A systematic literature review (SLR) was conducted in MEDLINE and Embase databases to identify studies in children and adults with DM and PM, published in the English language between Jan 1, 2011, and Apr 28, 2021. Only primary studies enrolling 10 or more patients were included, irrespective of country or region. Each eligible article was independently reviewed by two reviewers. The title and study abstracts were reviewed to assess eligibility for full-text review. The topics of interest were clinical, humanistic, and economic burden of disease, as well as current management and unmet needs in DM and PM. Information on geographic scope was extracted from the papers of included studies. The current abstract summarizes SLR results on humanistic burden of DM and PM in European patients. Results A total of 2,967 non-duplicated publications were retrieved from medical databases and analyzed against pre-defined study selection criteria. There were 2,574 records excluded at title and abstract screening. Remaining 393 records were analyzed in the full text with 208 papers considered relevant. Additional 21 papers were identified from searching reference list of relevant studies and conference proceedings. In total, 222 studies described in 229 publications were included in data abstraction. Among 43 studies conducted across 14 European countries, 12 studies evaluated health-related quality of life (HRQoL) and work productivity in patients with DM and PM. In 6 studies, patients received standard of care therapy. Six studies enrolled adults with DM and PM and 6 were conducted in patients with juvenile onset of DM. There were 6 cross-sectional analyses, 4 longitudinal cohort studies, 2 case-control studies, with sample size ranging from 11 to 246 patients. Adults with DM and PM had significantly worse HRQoL across multiple domains of 36-Item Short Form Survey (SF-36) compared to controls from general population, with a strong negative impact of muscle weakness on physical functioning. Patients reported high difficulties in performing leisure time activities, moving around and work as indicated by median scores of 4-5 points in a 7-point Myositis Activity Profile (MAP). Reduced grip force in DM and PM adults was significantly associated with worse performance in domestic activities in the MAP assessment (p&lt;0.05). In women with DM and PM, poor grip force additionally impacted vitality and mental health as measured by SF-36 (p&lt;0.05). There were no associations between grip force and any SF-36 domain in men. Approximately 60% of adult patients rated their ability to work as “poor” or “less good” according to the Work Ability Index, 68% of patients had more than one week of sick leave in the past year, and 20.8% of them were permanently not able to work for at least 2 years. Children and adolescents with DM had impaired physical and psychosocial functioning compared to healthy norms with 40% of individuals showing increased emotional distress requiring in-depth psychological assessment. Conclusion European patients with DM and PM experience a muscle weakness that has a detrimental impact on HRQoL, daily activities and ability to work. Similar disease impact on HRQoL was reported in patients in North America. These findings suggest a need for a novel therapy that will restore physical functioning in patients with DM and PM. Disclosure of Interests Jens Schmidt Speakers bureau: Euroimmun, CSL Behring, Consultant of: Alnylam, Argenx, Biotest, CSL Behring, Kezar Life Sciences, LFB, Novartis, Octapharma, UCB, Grant/research support from: CSL Behring, Novartis, Konrad Pisarczyk Consultant of: Kezar Life Sciences, Richard Leff Shareholder of: Kezar Life Sciences, Consultant of: Kezar Life Sciences, Kiruthi Palaniswamy Shareholder of: Kezar Life Sciences, Employee of: Kezar Life Sciences, Eunmi Park Shareholder of: Kezar Life Sciences, Employee of: Kezar Life Sciences, Li Long Shareholder of: Kezar Life Sciences, Employee of: Kezar Life Sciences